Polymorphic amyloid degeneration of the cornea: A Clinical and In vivo confocal microscopy study

Abstract

We report a rare case of polymorphic amyloid degeneration (PAD) with in vivo confocal microscopy and anterior segment optical coherence tomography (AS-OCT). A 74-year-old female presented to our service for suspected Fuchs corneal dystrophy. Her best-corrected visual acuity was 20/25 right eye (OD) and 20/20 left eye (OS). Slit-lamp biomicroscopy revealed bilateral punctate, refractile deposits in the deep stroma in an annular distribution with relative sparing of the central cornea. The remainder of the ocular examination was within expected limits. Central pachymetry was 546 μ OD and 535 μ OS. AS-OCT demonstrated focal hyper-reflective lesions in the deep stroma. Specular microscopy showed normal endothelial cell count and morphology. Confocal microscopy highlighted bright, enhancing punctate lesions in both the anterior stroma and deep stroma with normal intervening spaces. The patient was managed conservatively. Altogether, PAD is a rare, sporadically inherited condition that presents in the elderly. Confocal microscopy can aid in the diagnosis.